Linked Life Data

16226079

Source:http://linkedlifedata.com/resource/pubmed/id/16226079

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
4 Suppl
pubmed:dateCreated
2005-10-17
pubmed:abstractText
The short QT syndrome is a new congenital entity associated with familial atrial fibrillation and/or sudden death or syncope. Three different gain-of-function mutations in genes encoding for cardiac potassium channels (KCNH2, KCNQ1, and KCNJ2) have been identified up to now to cause short QT syndrome. The syndrome is characterized electrocardiographically by a shortened QTc interval less than 300 to 320 milliseconds and a lack of adaptation during increasing heart rates. During programmed electrical stimulation, atrial and ventricular effective refractory periods are shortened, and in a high percentage, ventricular tachyarrhythmias are inducible. Sudden cardiac death occurs in all age groups and even in newborns. Therapy for choice seems to be the implantable cardioverter-defibrillator because of the high incidence of sudden death. However, ICD therapy may be associated with an increased risk of inappropriate therapies for T wave oversensing, which, however, can be resolved by reprogramming ICD detection algorithms. The impact of sotalol, ibutilide, flecainide, and quinidine on QT prolongation has been evaluated. But only quinidine effectively suppressed gain-of-function in IKr, along with prolongation of the QT interval. Furthermore, in patients with a mutation in HERG (SQT1), quinidine rendered ventricular tachyarrhythmias noninducible and restored the QT interval/heart rate relationship toward a reference range. It may serve as an adjunct to ICD therapy or as possible alternative treatment especially for children and newborns.
pubmed:grant
pubmed:commentsCorrections
http://linkedlifedata.com/resource/pubmed/commentcorrection/16226079-10068851, http://linkedlifedata.com/resource/pubmed/commentcorrection/16226079-11104743, http://linkedlifedata.com/resource/pubmed/commentcorrection/16226079-11173780, http://linkedlifedata.com/resource/pubmed/commentcorrection/16226079-12925462, http://linkedlifedata.com/resource/pubmed/commentcorrection/16226079-14676148, http://linkedlifedata.com/resource/pubmed/commentcorrection/16226079-14678099, http://linkedlifedata.com/resource/pubmed/commentcorrection/16226079-15093889, http://linkedlifedata.com/resource/pubmed/commentcorrection/16226079-15159330, http://linkedlifedata.com/resource/pubmed/commentcorrection/16226079-15176418, http://linkedlifedata.com/resource/pubmed/commentcorrection/16226079-15569843, http://linkedlifedata.com/resource/pubmed/commentcorrection/16226079-15673388, http://linkedlifedata.com/resource/pubmed/commentcorrection/16226079-15761194, http://linkedlifedata.com/resource/pubmed/commentcorrection/16226079-15851347
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Oct
pubmed:issn
0022-0736
pubmed:author
pubmed:issnType
Print
pubmed:volume
38
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
75-80
pubmed:dateRevised
2011-9-26
pubmed:meshHeading
pubmed:year
2005
pubmed:articleTitle
Short QT syndrome. Genotype-phenotype correlations.
pubmed:affiliation
1st Department of Medicine-Cardiology, University Hospital Mannheim, Faculty of Clinical Medicine of the University of Heidelberg, 68167 Mannheim, Germany. martin.borggrefe@med.ma.uni-heidelberg.de
pubmed:publicationType
Journal Article, Review